Background

Extracranial brain tissue without a direct connection to the brain itself may be an isolated cutaneous embryonic defect, usually on occipital or parietal areas of the scalp. It is also known as heterotopic brain tissue or cutaneous ectopic brain (CEB).

Lee and Mclaurin
[1] described CEB in 1955 in a 1-year-old girl with a flat, almost perfectly circular, bluish-red plaque approximately 3 cm in diameter on the posterior midline of her scalp. Microscopically, heterotopic glial tissue in a pattern suggestive of abortive gyri and sulci was evident within the dermis. Additional patients with CEB have since been described.

Heterotopic brain tissue is a rare developmental abnormality that usually has no effect on neurological development.

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Pathophysiology

Cutaneous ectopic brain (CEB) may be an isolated embryonic rest or a congenital herniation through the skull with an eventual loss of connection. Perhaps the neural tube initially overgrows, preventing closure of the cranial or spinal coverings. Thus, its pathogenesis is uncertain.

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Epidemiology

Many congenital cutaneous disorders of the scalp have been described, all of which are quite uncommon. Cutaneous ectopic brain (CEB) is less common than encephalocele.

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Prognosis

The prognosis for patients with cutaneous ectopic brain (CEB) generally is good because no communication exists with underlying structures and few, if any, serious associated anomalies occur.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration